Pulmonary atresia with ventricular septal defect (PA-VSD) is a rare cyanotic congenital heart malformation characterized by underdevelopment of the right ventricular outflow tract and atresia of the pulmonary valve, ventricular septal defect (VSD) and pulmonary collateral vessels. Clinical features depend on the anatomic variability of the lesion and patients may be minimally symptomatic, severely cyanotic or may develop congestive heart failure. PA-VSD may represent a severe form of Tetralogy of Fallot (see this term).
The presence of a piece lung tissue which is not attached to the pulmonary blood supply and does not communicate with the other lung tissue (not connected to the standard bronchial airways and not performing a function in respiration).
Pulmonary atresia with ventricular septal defect and pulmonary sequestration.
Nakata T, Ikeda T, Doi H, Baba S, Sakata R. Asian Cardiovasc Thorac Ann. 2013;21(4):460-3.
A 5-day-old girl who had pulmonary atresia with ventricular septal defect, patent ductus arteriosus, intralobar pulmonary sequestration of the right lower lobe, and retroesophageal innominate vein, was referred to our institution.
